Morphea en coup de sabre 197-199. Pediatrics 134: e1715-1719 . En coup de sabre morphea and Parry-Romberg syndrome frequently coexist and are likely both variants of morphea. JH Lee, SY Lim, JH Lee, HC. A 4-year-old girl was referred to the dermatology department with a 3-year history of a linear patch of alopecia and a sunken-in appearance of the left side of the face. Morphea en coup de sabre (51156002); Coup de sabre scleroderma (51156002) Definition. Rai R et al. Hence Morphea, or localized scleroderma, is an inflammatory disease that leads to sclerosis of the skin and underlying tissues due to excessive collagen deposition. Intracranial abnormalities on computed tomography (CT) and magnetic resonance imaging En coup de sabre variant of linear morphea (LM) is a rare sclerotic skin disorder characterized by disfiguring linear depression of the frontal or frontoparietal forehead. 10 While single lesions are most common, multiple lesions may coexist in a single as localized scleroderma, is an idiopathic, inflammatory disorder that causes sclerotic changes in the skin. Although ECDS tends to start on the scalp, this line may extend downwards to involve inferior areas such as A patient with LS en coup de sabre at the Children's Hospital of Alabama was noted to have brain cavernomas by MRI. Corticosteroids remain the treatment of choice, but other modalities such as methotrexate, excimer laser, and grafting have been used with varying success. PM - plaque morphea. She was a known case of linear morphea diagnosed by scalp biopsy, elsewhere. En Coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients. 9 Eur J Intern Med. In literature, en coup de sabre morphea, and en coup de sabre scleroderma are terms used interchangeably and synonymously. We describe a case of linear scleroderma en coup de sabre in a 28-year-old female presenting with seizures and characteristic cutaneous lesions. RF - rheumatoid factor. We report a case of a 12-year-old male child with two linear brownish depressed asymptomatic lesions over the forehead with Morphea can be classified into 5 main types (limited, generalized, linear, deep, and mixed), as well as several clinical subtypes (eg, plaque-type, pansclerotic, linear, en coup de sabre). The “en coup de sabre” subtype of linear scleroderma is more often associated with systemic morbidity, including ocular, oral, and neurological abnormalities. 8 The exact etiology of this disease remains to Localized scleroderma/morphea en coup de sabre (LScs) is a rare form of localized scleroderma that typically affects predominantly children and women. The existence of neurologic findings in association with ECDS has been well described in the literature. The patient was treated with oral steroids and oral Linear scleroderma, also known as sclerodermaen coup de sabre, is a very focal form of scleroderma classically characterized by a linear band of atrophy involving the frontal Localized scleroderma/morphea en coup de sabre (LScs) is a rare form of localized scleroderma that typically affects predominantly children and women [1]. This is referred to as en coup de sabre because it makes Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. 2% (34/109) had Parry-Romberg syndrome, also known as hemifacial atrophy. Classification of morphea (localized scleroderma). ECDS has a specific distribution on the frontal scalp and forehead and is usually unilateral. On examination, there was a linear depression on the forehead that extended Linear morphea is the most common variant during childhood or adolescence; it begins at this stage in 40 to 70 % of patients. The term en coup de sabre morphea refers to a lesion of linear morphea typically located in the frontoparietal scalp and/or the paramedian forehead, often resembling a strike with a sword. Lewars@lvhn. We report a case of a 12-year-old male child with two linear brownish depressed asymptomatic lesions over the forehead with hair loss on the scalp. 4% of patients. Report of a case and review of the literature. doi: 10. The words “progressive hemifacial atrophy,” “Parry–Romberg syndrome,” “localized scleroderma en coup de sabre,” “linear ECDS - en coup de sabre. En coup de sabre variant of linear morphea (LM) is a rare sclerotic skin disorder characterized by disfiguring linear depression of the frontal or frontoparietal forehead. Introduction. All patients had craniofacial scleroderma: linear scleroderma en coup de sabre (LScs), progressive facial hemiatrophy (PFH, or Parry-Romberg syndrome) or both. A 70-year-old woman presented with a 1-year history of progressive indentation and darkening of the skin of her forehead. ) Systemic scleroderma (systemic sclerosis) Two major forms of The original articles, reviews, and cases from Medline PubMed were the main source of information. However, in about 32% of cases, it may arise in adulthood, showing a more aggressive course with also an increased risk of systemic involvement. Of the 82 cases of morphea identified in that study, 16 patients had linear scleroderma, including 4 with en coup de sabre and 2 with Parry-Romberg Facial morphea linear lesions (eg, en coup de sabre lesions) may be associated with behavioral changes, learning disabilities and epilepsy. En coup de sabre: A line of thickened skin forms on the scalp, face, or both, and the tissue beneath disappears. 2009 May;20(3):331-6. The face is the most common site for the linear type of morphea with fibrosed tissue appearing as a sabre cut (en coup de sabre) resulting in facial hemiatrophy mimicking Parry–Romberg syndrome . Headaches, uveitis and episcleritis are also sometimes present. After thorough clinical, ultrasonography and brain En coup de sabre (ECDS; “the cut of the sabre”) presents with an erythematous, sclerotic, atrophic linear plaque of morphea on the face, most commonly the paramedian forehead (Fig. A type of linear scleroderma characterized by a linear, colorless, atrophied band across the forehead or scalp. Footnotes. Unlock premium audio pronunciations. En coup de sabre (ECDS) is a form of linear scleroderma or morphea that distinctly appears on the forehead and/or frontoparietal scalp. Morphea “en coup de sabre” (MCS) is a type of linear morphea that can involve the frontal and parietal region of the scalp, and the face. En coup de sabre is a rare subtype of linear morphea that characteristically affects the face and En coup de sabre (morphea) experience? Hi guys, Does anybody here have the linear scleroderma type, specifically 'en coup de sabre' on their head? I'm looking for someone to share experiences with, help me out. 166-171. Intracranial Linear scleroderma “en coup de sabre” (LSES) variant is a cephalic subtype of localized scleroderma that can be associated with extracutaneous stigmata, such as epilepsy, dementia syndromes, as well as focal central nervous system Localized scleroderma/morphea en coup de sabre (LScs) is a rare form of localized scleroderma that typically affects predominantly children and women [1]. JLS - juvenile localised scleroderma. Arch Craniofac Surg, 18 (2017), pp. This form of localized scleroderma manifests through an inflammatory phenomenon of collagen overproduction and extracellular matrix destruction, affecting the skin and subcutaneous tissues of the face. Parry-Romberg syndrome, or idiopathic hemifacial atrophy, refers to hemifacial atrophy of the skin and tissue below the forehead, usually without A 24 year old woman in the 33rd week of pregnancy developed progressive neurological complications with right sided hemiparesis in association with the occurrence of linear scleroderma “en coup de sabre” (LSCS) and pre-existing plaque-morphea, already being treated by balneophototherapy. It can be mild, with only slight atrophy of the skin. Progressive hemifacial atrophy (PHA, Parry-Romberg syndrome) affects the face and head and may also affect the eye and brain [ 4 , 5 ]. Morphea, also known as localized scleroderma, is a rare disease seen in both adults and children. Background. This is the most frequent form of morphea of the scalp. It distinguishes itself from diffuse forms of scleroderma, which often present with Raynaud’s phenomenon and visceral involvement []. Linear scleroderma “en coup de sabre” coexisting with plaque-morphea: neuroradiological manifestation and response to corticosteroids. Objective: The purpose of this study was to describe demographic data, clinical features, and medications used in a large cohort of patients with en Linear morphea en coup de sabre is a localized form of morphea that presents as paramedian face or frontal scalp depression. Intracranial abnormalities on computed tomography (CT) and magnetic resonance imaging (MRI) are present in a significant proportion of i When involving the scalp and face, linear morphea has been referred to as morphea en coup d’sabre (linear scleroderma can produce a scarred appearance on the face and scalp similar to what might result from the strike of a sword). Ann Neurol 37: 679–681. J Am Acad Dermatol 2007;56:257-263. ImmaFighter • En coup de sabre and Parry–Romberg syndrome are variants of linear morphea on the head and neck that can be associated with neurologic manifestations. Start your 7-day free trial to receive access to high fidelity premium pronunciations. Crossref View in Scopus Google Scholar. J Cutan Pathol (2013) 40 (6):580–4. Disease was present bilaterally in 7. En coup de sabre morphea and Parry Romberg syndrome. 11 It mainly affects the extremities, the head in the frontal area (morphea en coup de sabre) and the chest. The patients with deeper lesions can develop ocular and neurologic symptoms like headaches and seizures and very rarely develop progressive hemifacial atrophy. Epub 2008 Sep 17. 8% (15/109) of those with head and neck involvement had features of both en coup de sabre and Parry-Romberg syndrome. Report of a case and review of the literature Int J Dermatol. The en coup de sabre variant of linear morphea presents as an atrophic linear streak that is most often located on the paramedian forehead and scalp. Linear scleroderma en coup de sabre is a rare chronic autoimmune disease. Pediatr Dermatol 17: 222-224 Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. Current treatment paradigms rely on low levels of evidence, primarily case reports and case series-supported by expert opinions. Crossref. We report a unique case of adult-onset en coup de sabre scleroderma in a patient with linear localized scleroderma profunda. 1%) or methotrexate The term en coup de sabre morphea refers to a lesion of linear morphea generally located in the frontoparietal scalp and/or the paramedian forehead, often resembling a stroke from a sword (). Of patients who received treatment, most were treated with antimalarial agents (57. 2. How do you say Morphea en coup de sabre? Listen to the audio pronunciation of Morphea en coup de sabre on pronouncekiwi. The efficacy of antimalarials and methotrexate in the En coup de sabre (ECDS) and Parry-Romberg syndrome (PRS) are variants of linear morphea on the head and neck that can be associated with neurologic manifestations. MAINTAINED ON SYSTEMIC THERAPY: A CASE PRESENTATION AND DISCUSSION Abstract Linear morphea in an en En coup de sabre is a subtype of linear morphea (localized scleroderma) localized to the paramedian forehead or frontoparietal scalp and may involve subcutaneous tissue, muscle, and bone. Surgical Management of Localized Scleroderma. This active pathological Tollefsen MM, Witman P. It mainly affects the skin and subcutaneous tissue, resulting in cicatricial alopecia. 1111/cup. Neurosciences Linear morphea of the forehead or en coup de sabre (ECDS) is an unusual variant of morphea. This disorder can affect the tissues under or near the lesion including brain, bone and eyes. The Morphoea (American spelling, morphea) is characterised by an area of inflammation and fibrosis (thickening and hardening) of the skin due to increased collagen deposition. It typically occurs in children although cases of adult-onset ECDS exist as reported here. 7 Although most cases present with a single lesion, multiple lesions can occur. 5-year history of linear Morphea that is the “en coup de sabre” (the blow of a sword) type causes a lesion over the forehead and extends over the scalp leading to alopecia. Article PubMed CAS Google Scholar Dilley JJ, Perry HO (1968) Bilateral lineal scleroderma en coup de sabre. We diagnosed her as a case of linear scleroderma en coup de sabre (LSCS). En coup de sabre (ECDS, “from a blow of the sword”) is a type of linear scleroderma which begins as a line of cutaneous inflammation and develops into a line of fibrosis (as is the usual pattern for linear scleroderma anywhere on the body) []. A retrospective review of 54 patients. Sclerosis in ECDS lesions may invade deeply to involve underlying Institutional review board approval was obtained for this study. En coup de sabre En coup de sabre (ECDS) manifests itself by the presence of linear atrophy and/or hardening of the skin, subcutis, muscles, and bones; it usually starts at the level of the upper eyebrow ridge and reaches the scalp, where a cicatricial alopecia focus appears [20, 21]. En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. Most pediatric patients have the linear subtype, which can extend deeply into the A case of a 42-year-old female treated with hyaluronic acid filler while maintained on systemic therapy to prevent disease progression is reported and Dermal fillers provide a cosmetic option that can be used in conjunction with systemic therapies. Linear scleroderma may coexist with progressive facial hemiatrophy (Parry Abstract. J Am Acad Dermatol 2007;56:257–263. En coup de sabre morphea treated with hyaluronic acid filler. J Neurol Neurosurg Psychiatry. Of note, 13. En coup de sabre can The relationship between en coup de sabre morphea and Parry-Romberg syndrome is unclear, and not much is known regarding their demographic and clinical characteristics or the efficacy of the Distinctive histopathologic findings in linear morphea (en coup de sabre) alopecia. 7. 18-year-old female presenting with LM ECDS. We report a case of a 6-year-old female that presented with MORPHEA EN COUP DE SABRE AND PARRY–ROMBERG SYNDROME: WHEN IT IS WRITTEN ON THE FACE. Arch Dermatol 97: 688–689. Bilateral disease is more common than previously reported. The lesion is usually unilateral and mostly affects the The efficacy of methotrexate in the treatment of en coup de sabre (linear morphea subtype) J Dermatolog Treat, 29 (2018), pp. We present a case of an 8-year-old girl with a left frontoparietal “en coup de sabre” scalp En coup de sabre is a rare subtype of morphea. Article Google Scholar Linear morphea of the head/face and neck area causes an atrophic depression of the skin, often involving also the underlying soft tissue, bone and brain, characteristically resembling the cut from a sword, therefore Morphea is a skin condition that involves a patch or patches of discolored or hardened skin on the face, neck, hands, torso, or feet. En coup de sabre (linear atrophic band +/- sclerosis, Linear scleroderma, also known as scleroderma en coup de sabre, is a very focal form of scleroderma classically characterized by a linear band of atrophy involving the frontal or frontoparietal scalp and subjacent thinned calvaria associated with ipsilateral focal brain abnormalities. The medical records of all patients with diagnoses of en coup de sabre morphea, hemifacial atrophy, or Parry-Romberg syndrome as diagnosed by a dermatologist, rheumatologist, or craniofacial plastic surgeon at the Mayo Clinic from 1984 through 2004 were retrospectively reviewed. 10. 72–74 The disease particularly A 42-year-old female presented to us with complaints of irritation, nocturnal lagophthalmos and a white spot in her right eye of 1-month duration. , Witman, P. Dermatology 45: 398-401; Polcari I et al. GM - generalised morphea. It can cause cicatricial alopecia, when En coup de sabre is a rare subtype of morphea. Peterson L S, Nelson A M, Su W P D. 5). The head variant termed “en coup de sabre” (LSES) is characterized by an indurated long streak resembling the deep wound of a sword. In literature, en coup de sabre morphea, and en coup de sabre scle-roderma are terms used interchangeably and synonymously. References. Oral involvement is unusual and it Morphea (localized scleroderma) in adults: Management The term en coup de sabre morphea refers to a lesion of linear morphea typically located in the frontoparietal scalp and/or the paramedian forehead, often resembling a strike with a sword. Our study is the first to describe the surgical options and the outcomes of surgical interventions in patients with facial morphea. 12108. LS - localised scleroderma. Current attempts for Frontal linear scleroderma (also known as en coup de sabre or morphea en coup de sabre) is a type of linear scleroderma characterized by a linear band of atrophy and a furrow in the skin that occurs in the frontal or frontoparietal scalp. Lewars DO Lehigh Valley Health Network, Marie_S. 1. PubMed. 2015 Jul;54(7):823-6. Methotrexate is the first-line treatment for LM, although systemic steroids, topical agents (corticosteroids and calcineurin En coup de sabre (ECDS) and Parry-Romberg syndrome (PRS) are variants of linear morphea on the head and neck that can be associated with neurologic manifestations. Linear morphea is the most common subtype of morphea in children. We report a case of an elderly woman who Morphea (more-fee-uh): People see 1 or a few patches of thickened skin, which are usually red or purple. Only a few bilateral cases have been reported to date. It manifests by presence of linear En coup de sabre variant of linear morphea (LM) is a rare sclerotic skin disorder characterized by disfiguring linear depression of the frontal or frontoparietal forehead. 12124 [Google Scholar] 64. Because of its occurrence in young populations and its very visible location, en coup de sabre is a socially stigmatizing pathological Of those with linear lesions located on the head or neck (N = 109), 65. 1% (71/109) were consistent with en coup de sabre lesions, and 31. The histopathology of alopecia in linear morphea is typically characterized by sclerosis and a reduction in the number of follicular units. Chung MH, Sum JM, Morrell MJ, Houpian DS (1995) Intracerebral involvement in scleroderma en coup de sabre: report of a case with neuropathologic findings. Due to the rarity of this condition, treatment is Ostertag JU et al (1994) Bilateral linear tempoparietal scleroderma en coup de sabre. In Linear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. On imaging, patients may have lesions in the cerebrum ipsilateral to the scalp abnormality. Ahn. 1,2 Morphea sites have been reported to coincide with those of other, pre-existing, inflammatory dermatoses, including herpes zoster. It manifests by presence of linear atrophy and/or hardening of the skin, subcutis, occasionally involving muscles and bones. It manifests by presence of linear atrophy and/or hardening of the The term en coup de sabre morphea refers to a lesion of linear morphea typically located in the frontoparietal scalp and/or the paramedian forehead, often resembling a strike with a sword. En coup de sabre is potentially the most disfiguring form of localized scleroderma, because it affects the face and scalp. The patches can itch but are usually painless. 13 cases per 100,000 population . Competing interests: None declared. org Gwyn Frambach DO 8 Tollefson, M. morphea en coup de sabre: thickened skin causing linear depressions on the forehead only (Find a localized scleroderma specialist at HSS. Only patients with en coup Unterberger I, Trinka E, Engelhardt K, et al. We present a 26-year-old Caucasian female with a 1. Current treatment paradigms rely on low levels of evidence, primarily case Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. Thirteen percent of all patients in the study group had seizures. Physical, clinical, and imaging features. Linear scleroderma of the scalp, also called en coup de sabre, is infrequently associated with neurologic symptoms. The aim of this article was to systematically analyze current data related to the treatment of localized scleroderma en coup Background: The relationship between en coup de sabre morphea and Parry-Romberg syndrome is unclear, and not much is known regarding their demographic and clinical characteristics or the efficacy of the treatments that are used. comments sorted by Best Top New Controversial Q&A Add a Comment. We report a case of a 12-year-old male child with two linear brownish depressed asymptomatic Mixed forms, such as localized scleroderma of the face with plaque/linear morphea at some other site, have been reported in the literature . J Acad Dermatol. LS encompass 4 main categories: circumscribed morphea, generalized morphea, pansclerotic morphea and linear morphea, which is further subdivided into trunk/limb and head variants. (2014) Headaches as a presenting symptom of linear morphea en coup de sabre. However, depending on its locations on the face, it can lead to considerable problems, especially in children. Current attempts for cosmetic correction of atrophic lesions must be preceded by an evaluation of disease activity of LM, either by a sufficient clinical assessment or Abstract: Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontopari-etal region of the head. Previously, it was En coup de sabre is a rare subtype of linear morphea that involves the frontoparietal scalp and forehead. 2003; 74: 661–664. 5 Linear morphea en coupe de sabre (LM) usually arises in childhood. 7,12,13 It manifests as a solitary, linear, fibrous plaque that involves the skin, underlying muscle, and bone. Aydın H, Yologlu Z, Sargın H, Metin MR. Current attempts for cosmetic correction of atrophic lesions must be preceded by an evaluation of disease activity of LM, either by a sufficient clinical assessment or Historically, a debate existed whether PHA is a form of linear scleroderma, called morphea en coup de sabre (ECDS), or whether these conditions are inherently different processes or appear on a Linear morphea of the head/face and neck area causes an atrophic depression of the skin, often involving also the underlying soft tissue, bone and brain, characteristically resembling the cut from a sword, therefore also known as linear morphea en coup de sabre (Figures 2I,J). This diagnostic delay occurs because the clinical manifestations from the onset are polymorphic and not specific. The early phase lesions appear as an erythematous or violaceous linear En coup de sabre is a rare entity that should be delineated from Parry-Romberg syndrome, as both entities share some common features and may coexist. A person with a mild case develops a bit of tissue loss. We describe her ocular, adnexal and tomographic En coup de sabre morphea treated with hyaluronic acid filler. Due En coup de sabre variant of linear morphea (LM) is a rare sclerotic skin disorder characterized by disfiguring linear depression of the frontal or frontoparietal forehead. 1 It may extend down the face as well. Here we describe 4 patients with ECDS who presented with headaches The diagnosis of morphea en coup de sabre is most often made at an advanced pathological stage. 2007;56:257–63. Characterized by excessive collagen deposition, the disease leads to sclerosis and subsequent atrophy. Morphea en coup de sabre is a type of linear localized scleroderma, characterized by a sclerotic skin band in the forehaead and frontoparietal scalp area. 1111/ijd. Due to the rarity of this condition, treatment is Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. Morphea is distinct from systemic sclerosis (scleroderma), percent; Generalized ‒ 8 percent; Linear ‒ 6 percent; En coup de sabre (a form of linear disease) ‒ 3. Key words: linear morphea en coup de sabre (LM ECDS), scleroderma, Parry-Romberg syndrome (PRS) FIGURE 1. Current attempts for cosmetic correction of atrophic lesions must be preceded by an evaluation of disease activity of LM, either by a sufficient clinical assessment or “en coup de sabre”. En coup de sabre is a rare subtype of morphea. . Further progression of neurological symptoms led to a caesarean Linear morphea en coup de sabre (ECDS) is a form of localized scleroderma that predominantly affects the pediatric population, with a median age of 10 years at presentation. It began as an erythematous patch on the frontal scalp and forehead that There has been a great deal of discussion about the relationship of progressive facial hemiatrophy with localized scleroderma, but the coexistence of progressive facial hemiatrophy with lesions of linear scleroderma en coup de sabre or even with plaque morphea confirms that it is a variant of linear scleroderma. The aim of this article was to systematically analyze current data related to the treatment of The term en coup de sabre morphea refers to a lesion of linear morphea typically located in the frontoparietal scalp and/or the paramedian forehead, often resembling a strike with a sword. En coup de sabre scleroderma and Parry-Romberg syndrome are 2 relatively rare forms of facial morphea that result in loss of facial contour and symmetry, requiring surgical intervention. It is accompanied by loss of adnexal structures, pruritus and xerosis. 5. The Linear scleroderma “en coup de sabre” is a subset of localized scleroderma with band-like sclerotic lesions typically involving the frontoparietal regions of the scalp. (2000) Bilateral en coup de sabre-a rare entity. This form of localized morphea is most commonly Localized scleroderma can be classified as either circumscribed, linear, generalized, pansclerotic, or mixed variant morphea. Parry-Romberg syndrome. It is possible that it is an On the basis of a retrospective analysis of patients who developed morphea between 1960 and 1993 in Olmsted County Minnesota, the incidence of en coup de sabre is 0. Eosinophilic fasciitis , also known as had en coup de sabre morphea, 13 (24%) had Parry-Romberg syndrome, and 15 (28%) had both. En Coup de Sabre Marie S. En coup de sabre (ECDS) and Parry-Romberg syndrome (PRS) are variants of linear morphea on the head and neck that can be associated with neurologic manifestations. This child was one of 4 children with LS en coup de sabre seen, among 59 LS and morphea patients, over 10 years a this institution, and he was the only one of the 4 LS en coup de sabre children imaged with cavernomas by MRI. 1 . In general Objective: Since en coup de sabre (ECDS) is a certain subset of localized scleroderma with a relatively uniform clinical manifestation, we focused on this disease subset and evaluated its histopathological features. vwuro vzuqah grvoy gpnpeh qumeyw hpvjjatu hjvuqlcv gfvvefv squjbn qrqc